Cerebral Palsy (CP).pdf

PAEDIATRICS AND CHILD HEALTH
• Paediatrics and Child Health
• Cerebral Palsy (CP)
Dr. Chongo Shapi (BSc.HB, MBChB)
- Medical Doctor
3/20/2022 Dr. Chongo Shapi, BSc.HB, MBChB, CUZ. 1

Definition
• Cerebral = cerebrum, Palsy = disorder of
movement
• Is a diagnostic umbrella term used to describe a
group of motor syndromes (posture, movement
and coordination) which is caused by a NON-
PROGRESSIVE “insult” to the motor control centres
of an immature brain (developing brain) usually
prenatally

• It is associated with:
1. Epilepsy
2. Abnormalities in:
a. Speech
b. Vision
c. Hearing
d. Swallowing (oromotor dysfunction = OMD),
hence FTT
e. Behaviour
f. Mental retardation
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Dr. Chongo Shapi, BSc.HB, MBChB, CUZ.
.
3

• Although historically considered a static
encephalopathy (loosely “brain paralysis”), this term
is not entirely accurate
• This is because of the recognition that the neurologic
features of CP often change or progress over time
• Many children and adults with CP function at a high
educational and vocational level
• These have no sign of the type of cognitive dysfunction
that is generally implied by the term encephalopathy
• CP is the leading cause of childhood disability affecting
function and development
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• CP is restricted to lesions of the brain only (CNS)
• Diseases specific to the peripheral nerves of the
spinal cord or to muscles although causing early
motor abnormalities, are not considered cerebral
palsy:
1. Peripheral nerves of the spinal cord e.g. spinal
muscular atrophy, myelomeningocele
2. Muscles e.g. muscular dystrophies

• The brain lesions of CP occur from the fetal or
neonatal period to up to the age of 3 years
• Insults to the brain after age 3 years may manifest
clinically as similar or identical to CP
• By definition, these lesions are not CP
• Despite the lesion to the developing brain
occurring before age 3 years, the diagnosis of CP
may not be made until after that time

• Some authorities advocate not making a
definitive diagnosis in selected cases until age 5
years or later
• This approach allows the clinical picture to be
clear and potentially allows exclusion of
progressive diseases
• In addition, some children have been diagnosed
with CP at an early age, only to have the
symptoms resolve later
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Aetiology of CP
• The causes are broad:
1. Developmental e.g. prematurity
2. Ischemic causes e.g. birth asphyxia
3. Infections (TORCHES)
4. Genetic causes
5. Metabolic causes
6. Other acquired aetiologies that produce a common
group of neurologic phenotypes
NB: Prematurity usually causes the spastic diplegic type
while birth asphyxia usually causes the athetoid type

Classification of CP
• Classification is according to resting tone and what
limbs are involved (called topographic
predominance)
• Is a combination of both anatomy and physiology:
1. Spastic (pyramidal) : 80%
- Damage is to the pyramidal tract (UMNL)
- Hence, there are UMNL signs
- Subtypes: (diplegia, hemiplegia, quadriplegia and
monoplegia)

Subtypes of spastic CP:
a. Diplegia: 30-40%
- Bilateral spasticity of the legs greater than in the arms
b. Hemiplegia: 20-30%
- Ipsilateral spasticity of both upper and lower limbs
- Upper extremity more than lower extremity
- E.g. right side involved with right arm more than right leg
c. Quadriplegia : 10-15%
- Marked motor impairment of all extremities
d. Monoplegia
- Rare
- Involvement is noted in 1 limb, either an arm or a leg
- If a patient has monoplegia, an effort should be made to rule
out causes other than CP

Classification of CP
2. Extrapyramidal (Athetoid, dyskinetic) : 10-15%
- Damage is to the basal ganglia
- Abnormal movements (athetosis, chorea,
bradykinesia/akinesia)
- Hypotonia and
- No UMNL signs
3. Ataxic/Hypotonic: < 5%
- Damage is to the cerebellum
- Truncal and extremity hypotonia with
- Hyperreflexia and persistent primitive reflexes
4. Mixed
- No single specific tonal quality predominating
- Typically characterized by a mixture of spastic and dyskinetic
components

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Risk factors for CP
1. Prenatal
- Pre-eclampsia
- Congenital malformations in the
fetus
- Polyhydramnios
- Maternal seizure disorder
- Male sex of fetus
- APH
- IUGR
- Multiple gestation
2. Perinatal
- Prematurity
- Birth asphyxia
- Chorioamnionitis
- Nonvertex and face presentation
of the fetus
3. Postnatal
- Infections (eg, meningitis,
encephalitis)
- Intracranial hemorrhage (eg,
due to prematurity, vascular
malformations, or trauma)
- Periventricular leukomalacia (in
premature infants)
- Hypoxia-ischemia (eg, from
meconium aspiration)
- Persistent fetal circulation
- Kernicterus

Clinical Manifestations
• Presentation is usually after failing to meet expected
growth motor developmental milestones or failing to
suppress obligatory primitive reflexes in the first year of life
• Abnormal muscle tone is the most frequently observed
symptom
• CP frequently manifests as early hypotonia for the first 6
months to 1 year of life, followed by spasticity
• The longer the period of hypotonia before hypertonia, the
greater the likelihood that the hypertonia will be more
severe
• Asymmetric crawling or failure to crawl
• Growth disturbance especially with FTT
• Review other systems to evaluate for multiple
complications that can occur with CP

Spastic diplegia
• Bilateral spasticity of the legs greater than in the
arms
• 1st often noted when an affected infant begins to
crawl
• Child uses the arms in a normal reciprocal fashion but
tends to drag the legs behind more as a rudder
(commando crawl) rather than using the normal four-
limbed crawling movement
• If the spasticity is severe, application of a diaper is
difficult because of the excessive adduction of the
hips
• Child may be unable to sit if there is paraspinal muscle
involvement

• Neurologic examination reveals UMNL signs
• If child is suspended by the axillae, a scissoring
posture of the lower extremities is maintained
• Walking is significantly delayed, feet held in a
position of equinovarus, and the child walks on
tiptoe
• Severe spastic diplegia is characterized by:
- Disuse atrophy
- Impaired growth of the lower extremities
- Disproportionate growth with normal
development of the upper torso

• Prognosis for normal intellectual development is
excellent for these patients
• Likelihood of seizures is minimal
• The most common neuropathologic finding is PVL
• This is in particular in the area where fibers
innervating the legs course through the internal
capsule
• MRI is very useful for evaluating the severity of
white matter injury and for excluding other brain
lesions

Spastic hemiplegia:
• Definite hand preference before age 1 year is a
red flag for possible hemiplegia
• The arm is often more involved than the leg and
difficulty in hand manipulation is obvious by 1 yr
of age
• Walking is usually delayed until 18–24 mo, and a
circumductive gait is apparent
• Examination of the extremities may show growth
arrest, particularly in the hand and thumbnail

• This is especially if the contralateral parietal lobe
is abnormal because extremity growth is
influenced by this area of the brain
• Spasticity is apparent in the affected extremities,
particularly the ankle, causing an equinovarus
deformity of the foot
• An affected child often walks on tiptoe because
of the increased tone
• Affected upper extremity assumes a dystonic
posture when the child runs

• UMNL signs may be present:
- Babinski sign
- Increased deep tendon reflexes
- Ankle clonus
- Weakness of the hand and foot dorsiflexors
• About 1/3rd of patients with spastic hemiplegia
have a seizure disorder
• This usually develops in the 1st year or 2
• About 25% have cognitive abnormalities including
mental retardation

• CT scan or MRI may show an atrophic cerebral
hemisphere with a dilated lateral ventricle
contralateral to the side of the affected extremities
• MRI is far more sensitive than CT for most lesions
seen with CP
• CT scan may be useful for detecting calcifications
associated with TORCHES infections
• Focal cerebral infarction (stroke) secondary to
intrauterine or perinatal thromboembolism is a vital
cause of hemiplegic CP
• The thromboembolism is related to thrombophilic
disorders, especially anticardiolipin antibodies

Spastic quadriplegia
• Is the most severe form of CP because of:
1. Marked motor impairment of all extremities and
2. High association with mental retardation and
seizures
3. Swallowing difficulties due to supranuclear bulbar
palsies, leading to aspiration pneumonia
4. Developmental disabilities
5. Speech and visual abnormalities
• The most common lesions seen on MRI scanning are
severe PVL and multicystic cortical encephalomalacia

• Neurologic examination shows UMNL signs
• Flexion contractures of the knees and elbows are
often present by late childhood
• Associated developmental disabilities, including
speech and visual abnormalities, are particularly
prevalent in this group of children
• Children with spastic quadriparesis often have
evidence of athetosis and may be classified as
having mixed CP

Athetoid or Choreoathetoid or Extrapyramidal CP
• Referred to in Europe as dyskinetic CP
• Characteristically hypotonic with poor head
control and marked head lag
• Develop increased variable tone with rigidity and
dystonia over several years
• Feeding may be difficult
• Tongue thrust and drooling may be prominent
• Speech is typically affected because the
oropharyngeal muscles are involved

• Absent of speech or sentences are slurred
• Deafness is common
• UMNL signs are not present
• Seizures are uncommon
• Intellect is preserved in many patients
• Extrapyramidal CP is the type most likely to be
associated with:
1. Birth asphyxia
2. Kernicterus
3. Metabolic genetic disorders e.g. mitochondrial
genetic disorders and glutaric aciduria

• Extrapyramidal CP secondary to birth asphyxia is
associated with bilaterally symmetric lesions in the
posterior putamen and ventrolateral thalamus
• The lesions appear to correlate the neuropathologic
lesion called status marmoratus in the basal ganglia
• MRI scan shows lesions in the globus pallidus
bilaterally

Complications of CP
MSS
Orthopaedic complications:
- Contractures leading to talipes equinovarus
- Hip dislocation
- Osteoporosis in wheelchair-bound patients or those with
ambulatory dysfunction
- Scoliosis
Integumentary
- Decubitus ulcers and sores
Respiratory system
- Aspiration pneumonia because of OMD
- Chronic lung disease/bronchopulmonary dysplasia
- Bronchiolitis/asthma

Complications of CP
GIT/Nutritional
- FTT (malnutrition) due to feeding and swallowing
difficulties secondary to OMD
- Patients may require a gastrostomy tube (G-tube)
or a jejunostomy tube (J-tube) to augment
nutrition
- Obesity less common than FTT
- Gastroesophageal reflux
- Constipation

Complications of CP
GIT/Nutritional
- Dental problems: dental caries, enamel
dysgenesis, malocclusion, and gingival
hyperplasia
- Increased incidence of dental problems is often
secondary to the use of medications, especially
drugs administered to premature infants and
antiepileptic agents

Complications of CP
Nervous system
- Epilepsy (15-60%), more in spastic quadriplegia
- Speech abnormalities
- Deafness (particularly in patients who had
kernicterus)
- Visual-field abnormalities due to cortical injury
- Strabismus

Nervous system
- Cognitive/psychologic/behavioral:
• Mental retardation (30-50%), most common with
spastic quadriplegia
• Attention-deficit/hyperactivity disorder (ADHD)
• Learning disabilities which has an impact on
academic performance and self-esteem
• Increased prevalence of depression
• Increased prevalence of progressive development
disorder or autism associated with concurrent
diagnosis of cerebral palsy

Diagnosis of CP
• The diagnosis of cerebral palsy is generally made
based on the clinical picture
• A thorough history and physical examination
should preclude a progressive disorder of the CNS
• This include degenerative diseases, metabolic
disorders, spinal cord tumour, or muscular
dystrophy
• However, some authors propose that the diagnosis
should be deferred until the child is age 2 years or
older

Diagnosis of CP
• Rule out anomalies at the base of the skull or
cervical spinal cord in patients with little
involvement of the arms or cranial nerves
• MRI or CT scans of the brain/spinal cord are
indicated to determine the location and extent of
structural lesions or associated congenital
malformations
• Audiometry and ophthalmological (visual acuity)
tests
• Genotyping should be considered in patients with
congenital malformations (chromosomes) or
evidence of metabolic disorders

Differential diagnosis
• Inherited metabolic disorders
• Hereditary spastic paraplegia
• Rett syndrome
• Metabolic myopathies
• Metabolic neuropathy
• Movement disorders in individuals with
developmental disabilities
• Traumatic peripheral nerve lesions
• Tumors of the conus and cauda equina
• Vascular malformations of the spinal cord

Investigations
• Neuroimaging
- Cranial USS, CT, MRI: intracranial haemorrhage, HIE,
PVL, hydrocephalus
- EEG (seizure disorder)
- Electromyography (EMG) and nerve conduction
studies
• Laboratory (not definitive)
- TFTs to rule out hypothyroidism: TSH, T4, T3
- Lactate and pyruvate: metabolic abnormalities
- Chromosomal analysis (Genotyping)

Treatment
Must be individualized based on the child's clinical
presentation and requires a multidisciplinary
approach:
Physiatrists Orthopaedic surgeons
Neurosurgeons Ophthalmologists
Gastroenterologists Geneticists
Nutritionist Pulmonologists
Occupational therapists Speech pathologists
Social workers Educators
Developmental psychologists

Treatment
• Patients need to be rehabilitated
• Rehabilitation is a comprehensive intervention
strategy designed to facilitate adaptation to and
participation in an increasing number and variety
of settings in a particular society and culture
• Neurologists and physiatrists play significant roles
in the management of anti-spasticity medications
• The paediatrician’s responsibility is to closely
supervise and manage the multiple medical
complications associated with cerebral palsy

Treatment
Physiatrist/Physiotherapists/Rehabilitation therapists
• Consulted for the evaluation and management of the
rehabilitation program
• Help with many aspects of care including spasticity
management
• May also administer IM botulinum toxin type A
Orthopaedic surgeons
• Correct any structural deformities
• Surgical management of hip dislocation, scoliosis, and
spasticity (eg, tenotomy, a tendon-lengthening
procedure)
• May also administer intramuscular botulinum toxin
type A

Treatment
Neurosurgeons
• Treat hydrocephalus, a tethered spinal cord, or spasticity
• Perform the dorsal rhizotomy procedure
Neurologists
• Help with differential diagnosis and with ruling out other
neurologic disorders
• Treatment of seizures
Geneticists
• May help with the differential diagnosis and with ruling out
other disorders
• To evaluate for an underlying genetic syndrome, particularly in
the setting of dysmorphic features, multiple organ
abnormalities, or a family history of a similar neurologic
syndrome
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Treatment
Gastroenterologist, nutritionist, and
feeding/swallowing team
• Manage feeding and swallowing difficulties and
GORD
• Assesses nutritional status so that child does not
suffer from growth failure or nutritional
deficiencies
Pulmonologists
• Manage chronic pulmonary disease due to
bronchopulmonary dysplasia and frequent or
recurrent aspiration

Treatment
Learning disability team
• Identifies specific learning disabilities, monitor cognitive
progression, and guide services through early intervention
and school
• Guide speech and language treatment and the use of
communicative devices
Others:
Ophthalmologists/ENT/dentists/endocrinologists
• Ophthalmologist: visual deficits
• ENT: hearing difficulties
• Dentists: dental checks up
• Endocrinologist: precocious puberty or treatment of
osteoporosis
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Medications in CP
• Antispasmodics/muscle
relaxants
- Bolinum toxin type A (Botox)
- Baclofen
- Benzodiazepines e.g. diazepam
- Dantrolene sodium
- Alpha 2- Adrenergic agonists
e.g. Tizanidine
• Anticholinergics (decrease
secretions and also tremors)
- Trihexyphenidyl (Atane)
• Anticonvulsants
- Phenobarbitone
- Levetiracetam
- Valproic acid
• Dopamine prodrugs
- Levodopa/carbidopa

Neurosurgery and Orthopaedic Surgery
• Intrathecal baclofen pump insertion
• Selective dorsal rhizotomy
• Stereotactic basal ganglia
• Orthopaedic surgical intervention

Selective dorsal rhizotomy
• Is a neurosurgical treatment that may be beneficial
in both the short term and long term to treat
velocity-dependent spasticity
• This procedure includes a laminectomy and then
surgical ablation of 70-90% of the dorsal or
sensory nerve roots
• Selective dorsal rhizotomy decreases spasticity by
decreasing reflexive motoneuron activation, which
is thought to result from the lack of descending
fiber input
• This surgery has come to be performed less
frequently since the advent of the baclofen pump

Selective dorsal rhizotomy
A: After laminectomy, the dura is opened and the dorsal spinal rootlets are exposed.
The rootlets are stimulated so that abnormal rootlet activity can be identified
B: A proportion of rootlets are transected

Thanks!

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